Selexipag as Add-on Therapy for Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: A Single-Center Retrospective Study

Purpose: This study examined the efficacy and safety of selexipag in treating pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD). Materials Methods: We conducted a retrospective patients CHD-associated PAH, treated since December 2017. Thirteen adult (mean age, 45.4 years; women, 77%) were as add-on therapy. Baseline characteristics, World Health Organization functional class, 6-minute walking distance (6MWD) test results, N-terminal pro-B-type natriuretic peptide levels, echocardiographic data, incidence side effects assessed. Results: The majority (12/13, 92.3%) experienced more than one treatment-associated complication; patient dropped out due to intolerable myalgia. results 6MWD (from 299.2 ± 56.2 m 363.8 86.5 m, p = 0.039) tricuspid regurgitation (TR) pressure gradient 84.7 20.5 mmHg 61.6 24.0 mmHg, 0.018) improved remained after treatment 12 patients. Based on non-invasive risk assessment, 8 (66.7%) showed improvement, 3 (25.0%) no interval change, status (8.3%) deteriorated. Moreover, compared low dosage, medium-to-high dosage greater increase (88.3 26.4 vs. 55.3 27.6 0.043) reduction TR (?33.7 10.9 ?12.5 12.0 0.015). Conclusion: Selexipag is an efficient vasodilator therapy PAH.